ANATOMICAL OBSERVATIONS OF THE PERSISTENT UROGENITAL SINUS IN VIRILIZED FEMALES DUE TO CONGENITAL ADRENAL HYPERPLASIA Mahmoud Marei, Ahmed Fares, Ayman Hussien, Hadeil Seif, Mona Mamdouh, Montasser El-Kottbi, Mohamed El-Barbary
Cairo University Specialized Paediatric Hospital, Egypt.
INTRODUCTION AND OBJECTIVES
RESULTS
Virilized females due to congenital adrenal hyperplasia present the most common form of female DSD. The anomaly is an external virilization resembling male genitalia and a persistent urogenital sinus.
Internal Anatomy: Urethral Length
Vaginal Depth 11
Number of Cases
12
9 6
Number of Cases
4
4 2
1 10mm
The length of the proximal urethra, measured from the bladder neck to the vaginal-urethral confluence is an important predictor of surgical complexity.
9
15mm
20mm
22-28mm
30-35mm
2
≤10mm
10-15mm
15-20mm
22-25mm
1
20-30mm
≥30mm
Mean vertical depth of the vaginalurethral confluence is 16 mm. 80% of cases (n = 21) occurred within one SD (11mm – 22mm).
Mean length of proximal prejunctional urethra is 22 mm. 60% of cases (n = 17) occurred within one SD (16.6mm – 28.5mm).
As the reconstruction is through a perineal approach, the vertical depth of the confluence from the perineum is similarly an important measurement. It determines the amount of the dissection required to deliver the vagina.
Confluence Depth (in millimeters)
35
30
Strong correlation between vaginal depth and proximal urethra
25
20
15
10
(r= ─0.78, p= 0.001)
5
0 0
5
10
15
20
25
30
35
40
Urethral Length (in millimeters)
PATIENTS AND METHODS
6
6
5
5
Prader Score (1 - 5)
There is no reported description of this level or depth, hence we present the measurements found in our series, and suggest a method to assess the difficulty of cases by applying a calculated ratio.
Prader Score (1 - 5)
Correlation of External Virilization (Prader’s score) to Internal Anatomy: No correlation with length of the proximal urethra (rs= ─0.035, p= 0.87) Weak correlation with depth of the confluence (rs= 0.27, p= 0.169)
4
3
2
1
Internal anatomy was assessed by: Length of the urethra proximal to the confluence Vertical depth of the confluence from the perineum.
2
0 0
Inclusion of Cases
3
1
0
We studied the anatomical details of the cases operated upon from 2011 to 2013.Twenty six cases were included.
4
5
10
15
20
25
30
35
40
0
Proximal Urethra (in millimeters)
5
10
15
20
25
30
35
Vaginal Depth (in millimeters)
Female (46,XX) DSD CAH
Assessment of External Anatomy by von Prader’s classification
Effect of Age: 18 months Age at Surgery: Median Range (Inter-Quartile) 6-78 months (14-30 months) Age has moderate correlation with vaginal depth (r= 0.45, p= 0.02), and weak correlation with urethral length (r= ─0.22, p= 0.28).
CONCLUSIONS
Genitography
The vaginal depth is an important indicator of the anatomical complexity, and is necessary to complete the preoperative assessment. The depth of the confluence is within 20mm in 85% of the cases.
The degree of external virilization does not significantly reflect the length of the urethra but could still hold some relationship with the depth of the vagina.
RECOMMENDATIONS We propose a ratio to neutralize the confounding variables, and render cases of different age easily comparable.
